An Unusual Presentation of Pulmonary SarcoidosisEmily Barsky, Harvard Medical School, Year IVGillian Lieberman, MD

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Our Patient: Ms. WS

35F with asthma, recurrent pneumonia, and remote history of intravenous drug use who presents with sudden onset right-sided chest pain and shortness of breath

Woke from sleep with sharp, stabbing chest pain

Seen in emergency department one week prior for chest pain, fevers, productive cough

Diagnosed with “multifocal pneumonia” and sent home with course of levofloxacin

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Our Patient: History

Review of Systems: Lesions on face and bilateral upper extremities for several months. Review of systems otherwise negative

Past Medical History: Asthma, diabetes, recurrent pneumonia

Family History: No family history of pulmonary disease, cancer, autoimmune disease

Social history: From Puerto Rico, moved to US in 1995, no recent travel. Lives in East Boston, homeless in past. Has positive TB contacts and prison contacts. Positive history of intravenous drug use but none in last 8 years, social alcohol use, 5 pack-year past smoking history. Negative HIV test 1-2 mos ago, no prior PPD.

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Our Patient: Physical Exam

Afebrile, HR 123, BP 114/60, Sp02 97% on 2L 02

Pulmonary: No breath sounds on right

Cardiac: No murmur

Skin: Erythematous, violacious plaques with scale on forehead, nose and at tattoo sites on upper extremities bilaterally

CXR was obtained given the patient’s acute dyspnea, chest pain, and hypoxia…

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Our Patient: Initial AP CXR

Image source: MGH, CAS

What are the findings?

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Pneumothorax

? Possiblelucency

MediastinalShift

Our Patient: Right Tension Pneumothorax

Collapsed lung tissue Depression

of R hemi- diaphragm

Mild rib splaying

Image source: MGH, CAS

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Pneumothorax: Types

SIMPLE

Small volume lung collapse (generally <30%)

Patient remains relatively asymptomatic

Signs on CXR:

White visceral line, marking lung/ pleural air interface; straight or convex towards chest wall

No pulmonary vasculature visible beyond this line

No mediastinal shift or occasionally mediastinal shift TOWARDS defect

TENSION

CLINICAL diagnosis

patient is symptomatic

usually only occurs with large pneumothoraces

One-way valve: air enters pleural space on inspiration but can’t exit on expiration--> pressure in pleural space builds up--> pleural pressure exceeds atmospheric pressure--> further lung compression--> respiratory failure

In extreme cases, can lead to cardiovascular collapse (impaired venous return to inferior and superior vena cava)

Signs on CXR:

Visceral pleural line with lack of pulmonary vasculature beyond that point

Mediastinal shift AWAY from defect (once pleural pressure sufficiently high)

+/- depression/inversion of ipsilateral hemidiaphragm

+/- splaying of ipsilateral ribsImage source: O’Connor AS, Morgan WE. Radiological Review of Pneumothorax. BMJ. 2005;330:1493-7.

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Pneumothorax: Classification

Primary Spontaneous Pneumothorax

Spontaneous rupture of a pleural bleb

Often occurs in thin, tall men

Tends to recur

Risk factors:

Smoking

Male gender

Family history

Marfan’s

Secondary Spontaneous Pneumothorax

Usually due to underlying lung disease. Can occur with any lung disease, but most commonly:

Chronic Obstructive Pulmonary Disease (70%)

Cystic Fibrosis

Mycobacterium Tuberculosis

Pneumocystis Carinii Pneumonia

Interstitial Lung Disease

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Pneumothorax: Management

Small pneumothorax:

If <30% hemithorax involvement and if clinically stable --> observe closely

Supplemental oxygen (hastens reabsorption)

Usually spontaneously resolve, occasionally requires needle aspiration

Large pneumothorax:

If >30% hemithorax involvement or clinically unstable --> requires treatment

Needle aspiration, chest tube placement (thoracostomy)

Tension pneumothorax:

Requires emergent chest tube placement

If with severe hemodynamic or respiratory compromise, can first decompress with subcutaneous needle, then place chest tube

What was done for our patient? Given the tension pneumothorax, a chest tube was placed immediately

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Our Patient: After Chest Tube Placement

1. Chest tube2. Small residual R pneumothorax3. Subcutaneous emphysema

AP View

Image source: MGH, CAS

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Our Patient: Work-Up

But why did our patient have a pneumothorax?

Let’s first look at her CXR from the week prior….

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Our Patient: Prior CXRPA plain film from one week PTA

Blurring of diaphragms R>L suggesting bibasilar atelectasis vs infiltrates

Ill-defined areas of opacification throughout both lung fields

o ?Lucency

Image source: MGH, CAS

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Our Patient: Prior CXR Cont’d

Focal area of opacity in anterior lung, butdifficult to ascertain which side it is on

Lateral view

Regardless, still no clear cause for tension pneumothorax…

So let’s do a CT

ED Conclusion one week prior: “ Multifocal pneumonia”

Image source: MGH, CAS

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Our Patient: Chest CT without Contrast

Residual pneumothorax

Subcutaneous emphysema

Cavitary lesion in RLL

Lung nodule

Probable Atelectasis

Axial ImageImage source: MGH, CAS

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Our Patient: Coronal and Sagital Reconstructions

RLL Cavity

Minor fissure

Major fissure

RLL nodule

Chest tube

Subcutaneous emphysema

Image source: MGH, CAS

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Our Patient: Lung Nodules

1.1 cm nodule in RLL

Sub-cm LLL nodule

Image source: MGH, CAS

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Our Patient: Bronchial Thickening

This is an axial cut superior to the location of the cavitary lesion

Notice the bronchial and peribronchial thickening on the right, as well as the micronodules and nodular opacities

Image source: MGH, CAS

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Our Patient: Lymphadenopathy o The soft tissue window allows for visualization of the mediastinum and detection of lymphadenopathy

o While the absence of contrast renders it suboptimal, there is fullness suggestive of mediastinal, hilar, and subcarinal lymphadenopathy

Coronal Reconstruction, Soft Tissue Window

Image source: MGH, CAS

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Our Patient: Summary of Findings

RLL cavitary lesion

Multiple nodules and micronodules

Bronchial and peribronchial thickening

Mediastinal, hilar and subcarinal adenopathy

However, her diagnosis is still unclear

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Cavitary Lung Lesions: Differential

Infection

Abscess (especially anaerobes, Nocardia, S. aureus, Klebsiella)

Mycobacterium (TB, nontuberculous)

Fungi

Septic emboli- endocarditis

Rheumatologic/Vasculitis

Most commonly Wegener’s

Others (rarely): Sarcoidosis, SLE, RA

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Cavitary Lung Lesions: Differential Cont’d

Neoplasm

Primary

Especially squamous cell carcinoma

Others: lymphoma, Kaposi’s Sarcoma

Metastatic (i.e. osteosarcoma)

Misc

Empyema, pulmonary embolism, Langerhan’s cell histiocytosis, bronchiolitis obliterans organizing pneumonia

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Our Patient: Work-up Cont’d

Given significant TB risk factors, induced sputum x 3 --> negative for acid fast bacilli

Bronchoscopy with bronchoalveolar lavage --> no organisms

But what about the skin findings?

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Companion Patient #1: Skin Lesions in Tattoo

Derm performed skin biopsy…

Path: granulomatous dermatitis

Our patient’s diagnosis? Sarcoidosis +/- lung superinfection

Image source: Lodha S, Sanchez M, et al. Sarcoidosis of the Skin: A Review for the Pulmonologist. Chest. 2009;136(2)583-96

Image source:Hatwin KE, Roddie ME. Pulmonary sarcoidosis: the ‘great pretender.’ Clinical Radiology. 2010 Aug;65(8)642-50

Skin biopsy in a similar patient

Skin lesions in a similar patient

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Sarcoidosis

Systemic granulomatous disease of unknown etiology affecting multiple organ systems

Epidemiology:

Usually presents between ages 20-40

Organ system involvement:

Lung >90%

Skin 25% (varied presentations)

Occular 20-50% (i.e. uveitis)

Liver (hepatitis ) 50-80%

Other organs systems: musculoskeletal (myositis, polyarthritis), reticuloendothelial (lymphadenopathy, hepatosplenomegaly), cardiac (arrhythmias), renal (focal segmental glomerulosclerosis, membranous nephropathy, crescentic), neurologic

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Sarcoidosis Cont’d

Signs and symptoms:

Up to 50% asymptomatic, diagnosed on routine CXR

1/3 have nonspecific symptoms (fever, malaise, fatigue, weight loss, decreased exercise tolerance)

Organ specific symptoms (i.e. cough, chest pain, dyspnea in the case of pulmonary sarcoid)

Diagnosis:1. Clinical and radiologic features consistent with

disease2. Noncaseating granulomas on histology

(transbronchial lung, skin, or lymph node biopsy most commonly)

3. Exclusion of other similar diseases

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Pulmonary Sarcoidosis: Stages

Stage 0: Normal CXR

Stage 1: Bilateral hilar adenopathy

Stage 2: Bilateral hilar adenopathy and reticular opacities/infiltrates (upper zones>lower zones)

Stage 3: Reticular opacities/infiltrates, resolved hilar adenopathy

Stage 4: Fibrosis (irreversible)

Image source: Wu JJ, Schiff KR. Sarcoidosis. Am Fam Physician. 2004 Jul 15;70(2):312-322.

Stage I Stage III Stage IV

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Pulmonary Sarcoidosis: Companion Patient #2

Notice the bilateral hilar adenopathy typical of early stage sarcoidosis

Also notice the reticulonodular opacities, most prominent in the RUL

PACS, BIDMCPA Plain film

Early Stage

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Pulmonary Sarcoidosis: Companion Patient #3

PACS, BIDMC

Extensive mediastinal and hilar lymph node calcification

Upper lobe volume lose secondary to fibrosis

Late Stage

Traction bronchiectasisMass-like area of fibrosis

PA Plain Film Coronal CT View

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Pulmonary Sarcoidosis: Other Radiological Findings

Nodules (multiple, often subpleural)

Bronchial wall thickening

Focal ground glass

Bronchovascular beading

Apical cysts

Honeycombing

Parenchymal masses or consolidation

Others: traction bronchiectasis, cavitation, pleural disease

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Pulmonary Sarcoidosis: Treatment and Prognosis

Treatment: Corticosteroids, cytotoxic agents, immunomodulators

Prognosis: Depends on host characteristics, organ involvement, disease extent

Spontaneous resolution in:

Stage 1: 90% of patients

Stage 2: 70% of patients

Stage 3: 20% of patients

Permanent functional impairment in 20-25%

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Sarcoidosis: Role of Imaging

CXR

Initial CXR with staging, prognostic power

Suboptimal for evaluating parenchymal abnormalities

High Resolution CT

Less prognostic power than CXR

Excellent for evaluating parenchymal abnormalities

Functional Imaging:

Can identify occult lesions, guide therapy for potential areas of reversible disease

PET/CT (FDG)- wave of the future?

excellent sensitivity, but significant radiation

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Functional Imaging of Sarcoidosis: FDG PET

Disseminated Sarcoidosis

Images from: Balan A, Hoey ET et al. Multi-technique imaging of sarcoidosis. Clinical Radiology 2010 Sep;65(9):750-60

R apical lung nodule Confirms splenic lesion

Spleen

R paratracheal, bilateral hilar and mediastinal lymph nodes

Pelvic lymph nodes

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Functional Imaging: Companion Patient #4

Image courtesy of Dr. Kevin Donohoe

Significant hilar, mediastinal, paratrachial, and cardiac involvement

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Our Patient: Outcome

Our patient was thought to have presumed sarcoidosis, with an ATYPICAL presentation (cavitary lung lesion and lung nodules, as well as more typical mediastinal and hilar lymphadenopathy), with probable superinfection

Chest tube was removed after several days and lung remained inflated

Patient was treated with empiric course of broad spectrum antibiotics for pulmonary superinfection

Outpatient follow-up was scheduled after completion of antibiotic course, with repeat CT to look for resolution of cavitary lesion and to better evaluate underlying lung parenchyma

The patient will likely start treatment for sarcoidosis at that time

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Summary I

Pneumothorax

There are two types of pneumothorax: simple and tension.

Tension pneumothorax can lead to respiratory and hemodynamic compromise and is a surgical emergency requiring immediate chest tube placement

When a patient presents with a primary spontaneous pneumothorax, consider further imaging to investigate the etiology

Cavitary lung lesions

There is a broad differential for cavitary lung lesions including infection, septic emboli, rheumatologic disease, and malignancy

Sarcoidosis can present with a cavitary lung lesion in rare instances

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Summary II

Sarcoidosis

Sarcoidosis is a systemic granulomatous disease, which most commonly affects the respiratory system

Pulmonary sarcoidosis has four stages of disease

Common pulmonary manifestations include hilar adenopathy and/or reticular opacities in early stages, and fibrosis in end stage disease

However, there are numerous other less common manifestations of pulmonary sarcoidosis (i.e. cavitation)

Imaging plays an important role in diagnosing, staging, and prognosticating for pulmonary sarcoidosis

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Acknowledgements

Mai-Lan Ho, MD

Marc Camacho, MD

Kevin Donohoe, MD

Pamela Mok, MD

Jennifer Son, MD

Iva Petkovska, MD

Gillian Lieberman, MD

Larry Barbaras

Emily Hanson

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BibliographyBalan A, Hoey ET, et al. Multi-technique imaging of sarcoidosis. Clinical Radiology. 2010

Sep;65(9):750-60.

Coker RK. Management strategies for pulmonary sarcoidosis. Ther Clin Risk Manag. 2009 Jun;5(3):575-84.

Gadkowski LB, Stout JE. Cavitary pulmonary disease. Clin Microbiol Rev. 2008 April;21(2);305-333.

Hatwin KE, Roddie ME, et al. Pulmonary sarcoidosis: the ‘great pretender.’ Clinical Radiology. 2010 Aug;65(8)642-50.

Hours S, Nunes H, et al. Pulmonary cavitary sarcoidosis. Medicine. 2008 May;87(3):142-51.

Mackie B, Humphrey DA, et al. Atypical sarcoidosis: a case report and literature review. Am J Med Sci. 2006;331(5):277-279.

O'Connor AR, Morgan WE. Radiological review of pneumothorax. BMJ. 2005 June; 330(7506):1493–1497.

Wu JJ, Schiff KR. Sarcoidosis. Am Fam Physician. 2004 Jul;70(2):312-322.